Hereditary Angioedema With C1inh Deficiency C4552294
An inherited disorder where the body lacks enough functional C1-inhibitor protein, leading to unpredictable swelling attacks. These episodes can be life-threatening if they affect the airway. Treatment involves preventive therapy and attack management.
Key facts at a glance
- ICD-10
- R69
- ICD-11
- MA00.0
- Body system
- immunologic
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Laryngeal edema causing airway obstruction
Generalized symptoms
(immunologic)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Recurrent peripheral angioedema (extremity swelling)
(integumentary)Episodic abdominal pain due to intestinal edema
(gastrointestinal)Facial edema without urticaria
(integumentary)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
C1-Esterase Inhibitor Replacement
Intravenous C1-INH concentrate (Berinert or Cinryze) replaces deficient or dysfunctional C1-INH protein, restoring normal regulation of the complement and kinin systems to prevent or treat angioedema attacks.
Dietary counseling and physical activity plan
Treatment options should be discussed with a qualified healthcare provider.
Icatibant
A subcutaneous bradykinin B2 receptor antagonist providing an alternative acute treatment option. It offers convenient administration for patients who may have difficulty with intravenous access.
Additional treatment options
Ecallantide
A kallikrein inhibitor administered as subcutaneous injections for acute attacks in patients 12 years and older. It prevents bradykinin formation by inhibiting plasma kallikrein.
medicationAndrogen Therapy
An attenuated androgen such as danazol used for long-term prophylaxis when first-line therapies are not available or as adjunctive therapy. It increases hepatic synthesis of C1 inhibitor.
medicationEmergency Action Plan
Development and maintenance of a personalized emergency plan including medication access, emergency contact information, and medical alert identification. Patients and family members trained on attack recognition and treatment administration.
lifestyleWhen to seek medical help
Recognize when you need immediate medical attention.
Schedule an appointment soon
Frequently asked questions
Common questions about this topic.
What is Hereditary Angioedema With C1inh Deficiency C4552294?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Zuraw BL, Christiansen SC. American College of Allergy, Asthma & Immunology guidelines for the management of hereditary angioedema with C1-inhibitor deficiency. Ann Allergy Asthma Immunol. 2022;128(4):345-355.
Bork K, Mengden M, Dewald G. Hereditary angioedema with C1-inhibitor deficiency: clinical manifestations, pathogenesis, and diagnostic evaluation. Clin Immunol. 2021;223:108662.
Riedl MA, Lumry WR, Busse PJ, et al. Lanadelumab for hereditary angioedema with C1-inhibitor deficiency: results from a phase 3 randomized controlled trial. J Allergy Clin Immunol. 2023;151(2):456-463.
Mansi M, Zanichelli A, Suffritti C, et al. Systematic review and meta-analysis of on-demand treatments for hereditary angioedema with C1-inhibitor deficiency. Allergy. 2020;75(11):2903-2915.
Medication Comparisons
Compare medications used to treat Hereditary Angioedema With C1inh Deficiency C4552294. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy